Cardiac Tumors

Cardiac Tumors

Overview

  • Cardiac Tumors

    • Classification:
    • Primary Tumors: 0.06% incidence
    • Secondary Tumors: 1.2% incidence

  • Primary Benign Tumors:

    • Constitute 80% of all cardiac tumors.
    • Examples include:
      • Myxoma (50% prevalence)
      • Papillary Fibroelastoma (15% prevalence, most common cardiac valve tumor)
      • Lipoma (15% prevalence)
      • Rhabdomyoma (most common benign cardiac tumor in children/newborns, 8% prevalence)
      • Fibroma (second most common pediatric cardiac tumor, 4% prevalence)
      • Hemangioma (2% prevalence)
      • Teratoma (1% prevalence)
      • Paragangliomas (may also be malignant)

Myxoma

  • Definition:
    • A benign primary cardiac tumor typically attached by a stalk (pedunculated) to the left side of the interatrial septum (80% of cases)
    • Rarely multicentric, can be friable, or located in the ventricles.
    • Large myxomas may obstruct blood flow or prolapse into the mitral or tricuspid annulus, mimicking stenosis.
    • Growth rate: approximately 1.8 cm per year.
Symptoms
  • Common Symptoms:
    • Dyspnea (positional)
    • Dizziness
    • Syncope
    • Palpitations
    • Chest pain
    • Fever
    • Weight loss
    • Joint pain
    • Clubbing
    • Malaise
    • Transient Ischemic Attack (TIA)/Cerebrovascular Accident (CVA)
    • Raynaud's phenomenon
    • Heart failure
Epidemiology
  • Familial Cases:
    • 10% associated with Carney syndrome, found more commonly in younger patients.
    • 65% of cases found in women.
Complications
  • Potential complications include:
    • Arrhythmias
    • Embolization (pulmonary or systemic)
    • Infection of the tumor
    • Edema
    • Sudden death
Diagnostics

  • Electrocardiogram:

    • May show atrial fibrillation/flutter.

  • Chest Imaging:

    • Chest X-ray/CMR/CCT/PET may reveal:
    • Cardiomegaly
    • Left/right atrial enlargement
    • Pulmonary edema
    • Unusual intracardiac calcification

  • Echocardiography:

    • Serial echocardiograms recommended to rule out recurrence (2-5% recurrence rate, usually within 48 months).
    • Tumor characteristics may include a globular, finely speckled mass with well-defined edges and pedunculated attachment.
    • Areas of tumor calcification may be visualized.
Treatment
  • Surgical Intervention:
    • Prompt complete surgical excision is necessary.
    • Recurrence possible if not completely excised.
    • Dacron patch may be needed for interatrial excision.
    • Mitral repair or replacement may be necessary.

Investigation Techniques for Myxoma

  • Echocardiographic Evaluation:

    • Describe chamber locations, size, shape, point of attachment, pattern of reflectivity, and motion characteristics.
    • Assess first-generation relatives of patients with familial myxomas.
    • Determine myxoma dimensions (length and width).
    • Visualize perfusion information (vascular channels or "myxoma blush").

  • Differentiation of Myxoma vs. Thrombus:

    • Myxomas are:
    • Usually mobile
    • Sharp demarcation with mottled appearance
    • Normal atrial dimensions
    • Normal atrioventricular valve.
    • Thrombus characteristics include:
    • Irregular surface
    • Layered, immobile
    • Broad-based attachment
    • Located near the posterior wall of the left atrium and associated with a dilated left atrium.
Recurrence and Malignancy Potential
  • Myxomas can reoccur (3% recurrence rate).
  • May exhibit some low-grade malignant features.
  • Rarely, 2D echocardiography may fail to detect highly vascularized myxomas.

Papillary Fibroelastoma

  • Also known as: cardiac papilloma, papillary fibroma, giant Lambl's excrescence, fibro-papilloma.
  • Epidemiology:
    • Most common valvular tumor in the heart (45% on aortic valve).
    • Occurs with equal frequency in both sexes, usually found in patients older than 60 but has been reported in individuals from 9 months to 92 years.
  • Clinical Manifestations:
    • Symptoms include dyspnea, cyanosis, peripheral/pulmonary emboli, transient ischemic attacks, visual changes, and possible heart failure.
  • Locations:
    • Often attached to atrioventricular valves, semilunar valves, left ventricular outflow tract, or rarely on supportive structures such as papillary muscles or chordae tendineae.
  • Size:
    • Rarely exceeds 1 cm in diameter, typically gelatinous in appearance.
  • Asymptomatic Cases:
    • Small left-sided non-mobile types may not require surgical resection.
  • Indications for Surgical Resection:
    • Large (> 1.0 cm), mobile types or in younger patients.
Imaging Appearance
  • 2D Echocardiography:
    • Appear as small, mobile, pedunculated echo-dense mass.
    • May mimic vegetation; differentiation may require clinical information.
  • Doppler Studies:
    • May demonstrate no abnormalities, but significant regurgitation may be observed.
    • Transesophageal echocardiography (TEE) may be more helpful in identifying features for surgical removal.

Lipoma

  • Circumscribed, encapsulated tumor, usually solitary.
  • Most frequently located in the left ventricle, right atrium, or interatrial septum.
  • Sizes may vary but most are intramuscular, epicardial, or subendocardial.
  • Symptoms:
    • Patients are usually asymptomatic, though may cause conduction disturbances and pericardial effusions.
  • Treatment:
    • Surgical excision may be indicated.

Lipomatous Hypertrophy of the Interatrial Septum

  • Represents non-encapsulated hyperplasia of adipose tissue in the anterior atrial septum.
  • Associated with:
    • Older age, obesity, diabetes, supraventricular rhythm, conduction disturbances, and sudden cardiac death.
  • Diagnosis involves echocardiography showing thickened intra-atrial septum (commonly ranging from 1.5cm to 3cm thick).
  • Surgical Considerations:
    • Indications may arise in cases of significant symptoms or arrhythmias.

Fibroma

  • Usually ventricular, commonly intramural and solitary.
  • Most frequently found embedded in the myocardium, particularly anterior wall of the left ventricle.
  • Clinical Symptoms:
    • Heart failure, sudden death, ventricular tachycardia, atypical chest pain.
  • Surgical intervention often complicated by size; cardiac transplantation may be required in nonresectable cases.
  • Echo Findings:
    • Identified through apical/subcostal views, may appear highly refractile in echocardiography.

Rhabdomyoma

  • Most common cardiac tumor found in children, associated with tuberous sclerosis.
  • Occurs equally in both ventricles, most often during the first year of life.
  • Symptoms include arrhythmia, AV block, and may cause significant obstruction.
  • Often spontaneously disappear.
  • Treatment:
    • May involve antiarrhythmic drugs or surgical resection.

Primary Malignant Intracardiac Tumors

  • Types Include:
    • Angiosarcoma: Commonly found in right atrium (28%).
    • Rhabdomyosarcoma: Often infiltrates ventricular myocardium (11%).
    • Fibrosarcoma: Extensive infiltration common (8%).
    • Osteosarcoma: Usually in left atrium (7%).
    • Malignant fibrous histiocytoma: Often in left atrium (6%).
    • Lymphoma: Commonly found in right atrium (6%).
  • Clinical Presentation:
    • Symptoms may include chest pain, arrhythmias, and rapid disease progression individual cases averaging a mean survival of 10 months.

Secondary Tumors (Metastatic)

  • 20 to 40 times more common than primary tumors.
  • Cardiac metastasis occurs in approximately 5% of patients who died of malignant tumors.
  • Clinical presentation often includes pericardial effusion and symptoms of neoplastic disease.
  • Common Primary Tumors:
    • Melanoma (50-65%), bronchiogenic carcinoma, breast cancer, lymphoma, renal cell carcinoma, etc.
  • Diagnostics:
    • May show pericardial effusion, wall thickening, or protrusion into cardiac chambers.

Pericardial Cysts

  • Most common benign tumor of the pericardium, occurring in 1 per 100,000 individuals.
  • Ranges in size from 2 to 16 cm.
  • Symptoms may include chest pain or dyspnea.
  • Most often found in the right costophrenic area, but may occur in other locations.
  • Imaging Outcome:
    • Requires needle aspiration for evaluation; may resemble other tumors of the pericardium.

Extracardiac Tumors

  • May include mediastinal cysts, hematomas, or other such structures that can displace the heart or obstruct cardiac chambers.

Carcinoid Heart Disease

  • Definition:
    • Results from metastasizing carcinoid tumors, frequently located in the ileum or other areas.
  • Symptoms:
    • Cutaneous flushing, intestinal hypermobility, bronchial constriction, heart failure, and unique cardiac lesions.
  • Commonly affects the right heart more than left; cardiac lesions 50% of patients show involvement.
  • Treatment:
    • Medical and surgical treatments aimed at managing symptoms and possibly removing tumors if non-metastatic.